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Sydenham舞蹈病

Author
Donald L Gilbert, MD, MS
Section Editors
Marc C Patterson, MD, FRACP
Sheldon L Kaplan, MD
Thomas JA Lehman, MD
Deputy Editor
Carrie Armsby, MD, MPH
Translators
杨欣伟, 副主任医师，副教授

引言

Sydenham舞蹈病(Sydenham chorea, SC)也称为风湿性舞蹈病，是急性风湿热(acute rheumatic fever, ARF)的主要临床表现之一，也是儿童期最常见的获得性舞蹈病类型。该病是一种运动障碍，特征为舞蹈样动作(肢体和面部的短暂、随机、不规则的不自主运动)、情绪不稳和肌张力低下。

本专题将总结SC的临床表现、诊断和治疗。ARF的其他临床表现及风湿热的诊断和治疗方法参见其他专题。 (参见“Acute rheumatic fever: Clinical manifestations and diagnosis”和“急性风湿热的治疗与预防”)

病理生理学

虽然SC与A组链球菌(group A streptococcal, GAS)感染明确相关[1]，但其发病机制尚未完全明确。目前认为分子模拟发挥重要作用，在该机制中，针对部分A组链球菌的抗体与易感受试者的宿主抗原发生交叉反应。

在ARF中，抗体针对的是N-乙酰-β-D-氨基葡萄糖(N-acetyl-beta-D-glucosamine, NABG或GlcNAc)，NABG是A组链球菌的免疫优势糖类抗原。这些抗体很可能在风湿性心脏炎的瓣膜损伤和ARF的其他表现中有一定的作用(参见“急性风湿热的流行病学和发病机理”，关于‘分子相似性’一节)。

不同的NABG抗体亚群似乎与ARF不同的临床表现相关。在SC中，抗体在神经元细胞表面与溶血神经节苷脂结合[2,3]，在此处它们能够触发信号级联[4]。这些抗体也能识别细胞内的微管蛋白[5]。在无SC的ARF患者或SC痊愈的患者中，均未发现微管蛋白特异性抗体。编码这些抗体的基因与编码参与运动神经病发病机制的抗体的基因相似[6]。因此，微管蛋白似乎是SC发病机制中重要的神经元靶点。

                       

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Literature review current through: 2017-06 . | This topic last updated: 2016-09-02.
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